title: Contractures of the lower extremities in spinal muscular atrophy type II. Descriptive clinical study with retrospective data collection.
abstract: BACKGROUND
  Early development of contractures and progressive scoliosis with pelvic obliquity are the most significant orthopaedic problems for children with the intermediate form of spinal muscular atrophy, SMA type II. This study deals with the restrictions of the passive range of motion and the development of contractures of the lower extremities in these patients.
  
  
  METHODS
  We followed up 143 patients, 74 female and 69 male, with SMA type II for an average of 5.3 years (± 4.0, 0.2 - 18.7). Their average age at the first examination was 8.4 years (± 6.6, 0.1 - 34.1) and at the last examination 12.3 years (± 6.5, 0.7 - 37.1). The passive range of joint motion was determined using a goniometer. According to Johnson et al. (1992), we calculated the relative contracture index (CI).
  
  
  RESULTS
  The loss of range of motion (ROM) and the contractures of the joints of the lower extremities (hip, knee and ankle) develop early and increase progressively with age. Marked restriction of motion with severe flexion contractures in some cases was observed in the knee followed by the hip and ankle joint.
  
  
  CONCLUSIONS
  The findings of this study give us more information about the development of contractures and deformities of the joints of the lower extremities and aim to help to improve the quality of orthopaedic care of patients with SMA type II.
authors.affiliation: Department of Orthopaedic Surgery, Friedrich-Alexander-University Erlangen-Nuremberg, Germany. A.Fujak@t-online.de
authors.forename: Albert
authors.initials: A
authors.lastname: Fujak
authors.forename: Carsten
authors.initials: C
authors.lastname: Kopschina
authors.forename: Florian
authors.initials: F
authors.lastname: Gras
authors.forename: Raimund
authors.initials: R
authors.lastname: Forst
authors.forename: Jürgen
authors.initials: J
authors.lastname: Forst
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003286:Contracture / Q000209:etiology*; D005260:Female; D006801:Humans; D035002:Lower Extremity; D008297:Male; D012189:Retrospective Studies; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications*